The Neurological Masquerade of Intravascular Lymphomatosis

Abstract

Intravascular lymphomatosis is an uncommon disease with a myriad of potential neurological manifestations. Diagnosis requires a high index of suspicion and a pathological examination. If diagnosed early, aggressive chemotherapy is potentially curative, although the overall prognosis remains dismal.

Unveiling the Clinical and Imaging Signatures of Intravascular Lymphoma of the Central Nervous System: A Multicentric Cohort Study

“…These 2 clinical features are not exclusive and could be combined at individual level as previously observed. 7 Third, overall, men and women seem to be equally affected but at a fairly advanced age, with an average age at onset around 65 years in line with previous data of the literature. 1,2,15,16 The analysis of MRI results reveals several original aspects of cerebral IVLBCL that could help diagnose the disease.…”

“…However, most of these investigations, conversely to the present study, were not specifically dedicated to the analysis of IVLBCL involving the CNS 3,15,16 . The largest comparable study was a monocentric case series of 8 patients 7 showing data in line with most of our findings and reinforcing the reliability of the present results. This study has also multiple strengths.…”

“…They include “stroke like‐episodes” with frequent fluctuating focal symptoms developing over minutes or days that can lead to more or less persisting focal deficits, or a picture mimicking an encephalopathy with rapidly progressive cognitive deterioration. These 2 clinical features are not exclusive and could be combined at individual level as previously observed 7 . Third, overall, men and women seem to be equally affected but at a fairly advanced age, with an average age at onset around 65 years in line with previous data of the literature 1,2,15,16 …”

Unveiling the Clinical and Imaging Signatures of Intravascular Lymphoma of the Central Nervous System: A Multicentric Cohort Study

“…These 2 clinical features are not exclusive and could be combined at individual level as previously observed. 7 Third, overall, men and women seem to be equally affected but at a fairly advanced age, with an average age at onset around 65 years in line with previous data of the literature. 1,2,15,16 The analysis of MRI results reveals several original aspects of cerebral IVLBCL that could help diagnose the disease.…”

“…However, most of these investigations, conversely to the present study, were not specifically dedicated to the analysis of IVLBCL involving the CNS 3,15,16 . The largest comparable study was a monocentric case series of 8 patients 7 showing data in line with most of our findings and reinforcing the reliability of the present results. This study has also multiple strengths.…”

“…They include “stroke like‐episodes” with frequent fluctuating focal symptoms developing over minutes or days that can lead to more or less persisting focal deficits, or a picture mimicking an encephalopathy with rapidly progressive cognitive deterioration. These 2 clinical features are not exclusive and could be combined at individual level as previously observed 7 . Third, overall, men and women seem to be equally affected but at a fairly advanced age, with an average age at onset around 65 years in line with previous data of the literature 1,2,15,16 …”

A Progressive Multifocal Neurological Syndrome in a 42‐year‐old Woman

“…A better prognosis is seen with the cutaneous variant (2). Transient clinical improvement on steroid therapy has been reported in the literature (4) and it can be dramatic (1) and, perhaps, this played some role in prolonging survival in our case. Disseminated disease was not evident in our case, which may also have impacted on survival.…”

Intravascular lymphoma presenting as a longitudinally-extensive myelitis: Diagnostic challenges and etiologic clues

“…PNS involvement is rare: a radiculopathy, polyradiculopathy, cauda equina syndrome, peripheral neuropathy, or myopathy have all been described [5,8,[12][13][14][15][16][17][18][19]. As in our case, a subacute onset progressive paraparesis due to spinal cord involvement may be the presenting manifestation of the disease [10,20,21]. A predilection for the conus medullaris has been reported syndrome [8,16,20,22].…”

Unveiling the Clinical and Imaging Signatures of Intravascular Lymphoma of the Central Nervous System: A Multicentric Cohort Study

“…These 2 clinical features are not exclusive and could be combined at individual level as previously observed. 7 Third, overall, men and women seem to be equally affected but at a fairly advanced age, with an average age at onset around 65 years in line with previous data of the literature. 1,2,15,16 The analysis of MRI results reveals several original aspects of cerebral IVLBCL that could help diagnose the disease.…”

“…However, most of these investigations, conversely to the present study, were not specifically dedicated to the analysis of IVLBCL involving the CNS 3,15,16 . The largest comparable study was a monocentric case series of 8 patients 7 showing data in line with most of our findings and reinforcing the reliability of the present results. This study has also multiple strengths.…”

“…They include “stroke like‐episodes” with frequent fluctuating focal symptoms developing over minutes or days that can lead to more or less persisting focal deficits, or a picture mimicking an encephalopathy with rapidly progressive cognitive deterioration. These 2 clinical features are not exclusive and could be combined at individual level as previously observed 7 . Third, overall, men and women seem to be equally affected but at a fairly advanced age, with an average age at onset around 65 years in line with previous data of the literature 1,2,15,16 …”

A Progressive Multifocal Neurological Syndrome in a 42‐year‐old Woman

“…A better prognosis is seen with the cutaneous variant (2). Transient clinical improvement on steroid therapy has been reported in the literature (4) and it can be dramatic (1) and, perhaps, this played some role in prolonging survival in our case. Disseminated disease was not evident in our case, which may also have impacted on survival.…”

Intravascular lymphoma presenting as a longitudinally-extensive myelitis: Diagnostic challenges and etiologic clues

“…PNS involvement is rare: a radiculopathy, polyradiculopathy, cauda equina syndrome, peripheral neuropathy, or myopathy have all been described [5,8,[12][13][14][15][16][17][18][19]. As in our case, a subacute onset progressive paraparesis due to spinal cord involvement may be the presenting manifestation of the disease [10,20,21]. A predilection for the conus medullaris has been reported syndrome [8,16,20,22].…”

Unveiling the Clinical and Imaging Signatures of Intravascular Lymphoma of the Central Nervous System: A Multicentric Cohort Study

“…These 2 clinical features are not exclusive and could be combined at individual level as previously observed. 7 Third, overall, men and women seem to be equally affected but at a fairly advanced age, with an average age at onset around 65 years in line with previous data of the literature. 1,2,15,16 The analysis of MRI results reveals several original aspects of cerebral IVLBCL that could help diagnose the disease.…”

“…However, most of these investigations, conversely to the present study, were not specifically dedicated to the analysis of IVLBCL involving the CNS 3,15,16 . The largest comparable study was a monocentric case series of 8 patients 7 showing data in line with most of our findings and reinforcing the reliability of the present results. This study has also multiple strengths.…”

“…They include “stroke like‐episodes” with frequent fluctuating focal symptoms developing over minutes or days that can lead to more or less persisting focal deficits, or a picture mimicking an encephalopathy with rapidly progressive cognitive deterioration. These 2 clinical features are not exclusive and could be combined at individual level as previously observed 7 . Third, overall, men and women seem to be equally affected but at a fairly advanced age, with an average age at onset around 65 years in line with previous data of the literature 1,2,15,16 …”

A Progressive Multifocal Neurological Syndrome in a 42‐year‐old Woman

“…A better prognosis is seen with the cutaneous variant (2). Transient clinical improvement on steroid therapy has been reported in the literature (4) and it can be dramatic (1) and, perhaps, this played some role in prolonging survival in our case. Disseminated disease was not evident in our case, which may also have impacted on survival.…”

Intravascular lymphoma presenting as a longitudinally-extensive myelitis: Diagnostic challenges and etiologic clues

“…PNS involvement is rare: a radiculopathy, polyradiculopathy, cauda equina syndrome, peripheral neuropathy, or myopathy have all been described [5,8,[12][13][14][15][16][17][18][19]. As in our case, a subacute onset progressive paraparesis due to spinal cord involvement may be the presenting manifestation of the disease [10,20,21]. A predilection for the conus medullaris has been reported syndrome [8,16,20,22].…”